Description: Homo sapiens von Hippel-Lindau tumor suppressor-like (VHLL), mRNA. RefSeq Summary (NM_001004319): Von Hippel-Lindau (VHL) tumor suppressor protein is a component of an E3 ubiquitin ligase complex that selectively ubiquitinates the alpha subunit of the hypoxia-inducible factor (HIF) transcription factor for proteasome-mediated degradation. Inactivation of VHL causes VHL disease and sporadic kidney cancer. This gene encodes a VHL homolog that lacks one of two key domains necessary for VHL function. This gene may contribute to the regulation of oxygen homeostasis and neovascularization during placenta development. This gene is intronless, and can also be interpreted as a retrotransposed pseudogene of the VHL locus located on chromosome 3. However, the protein is represented in this RefSeq due to evidence in PMID:14757845 that strongly suggests it is translated. The same publication also indicates that this protein binds HIF alpha but fails to recruit the E3 ubiquitin ligase complex, and it therefore functions as a dominant-negative VHL protein and a protector of HIF alpha. [provided by RefSeq, Jan 2010]. Transcript (Including UTRs) Position: hg19 chr1:156,268,415-156,269,428 Size: 1,014 Total Exon Count: 1 Strand: - Coding Region Position: hg19 chr1:156,268,561-156,268,980 Size: 420 Coding Exon Count: 1
ID:VHLL_HUMAN DESCRIPTION: RecName: Full=Von Hippel-Lindau-like protein; Short=VHL-like protein; Short=VLP; FUNCTION: Functions as a dominant-negative VHL to serve as a protector of HIFalpha. SUBUNIT: Interacts via the beta domain with the ODD domain of HIF1A This interaction is independent of prolyl hydroxylation of HIF1A. TISSUE SPECIFICITY: Abundantly expressed in the placenta. MISCELLANEOUS: Has little or no E3 ubiquitin ligase activity as it lacks the alpha domain required for nucleating the multiprotein E3 ubiquitin ligase complex.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q6RSH7
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
